Skip to main content
Log in

Prise en charge médicale per- et postnatale de la hernie congénitale diaphragmatique

Perinatal and postnatal management of congenital diaphragmatic hernia

  • Mise au Point / Up to Date
  • Published:
Revue de médecine périnatale

Résumé

Malgré les progrès de la réanimation, la mortalité néonatale des enfants porteurs de HCD reste élevée, proche de 30–40 %, essentiellement du fait de l’hypoplasie pulmonaire et d’une hypertension artérielle pulmonaire. Les principales séquelles observées sont respiratoires (HTAP chronique, dysplasie bronchopulmonaire, susceptibilité aux infections virales), digestives (reflux gastro-oesophagien, trouble de l’oralité), nutritionnelles (dénutrition d’origine multifactorielle: RGO, trouble de l’oralité, insuffisance respiratoire) et orthopédiques (scoliose). La prise en charge actuelle de ces complications est tardive: elle nécessite une intervention plus précoce, qui doit débuter dès la période néonatale. Les objectifs de la prise en charge médicale seront: 1) d’assurer une oxygénation tissulaire et la décarboxylation tout en minimisant le baro-volotraumatisme du poumon; 2) d’assurer une fonction circulatoire adéquate en limitant les conséquences de l’HTAP; 3) de prévenir la morbidité respiratoire et digestive. Elle nécessite impérativement un suivi et une prise en charge multidisciplinaire spécialisée, du fait de l’intrication de ces différentes complications.

Abstract

Despite improvements in intensive care, lung hypoplasia and persistent pulmonary hypertension cause a high mortality rate in newborns with CDH. Sequelae including bronchopulmonary dysplasia, chronic pulmonary hypertension, gastro-oesophageal reflux, growth retardation and oral aversion are observed in half the surviving infants. The aims of post-natal management are: 1) to provide adequate tissular oxygenation and decarboxylation, with minimal lung volo-or barotrauma; 2) to optimise circulatory function through PPHN management; 3) to prevent respiratory and nutritional morbidity. Long-term follow-up and multidisciplinary management are required.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Références

  1. Skari H, Bjornland K, Haugen G, et al (2000) Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J Pediatr Surg 35:1187–1197

    Article  PubMed  CAS  Google Scholar 

  2. Slavotinek AM (2004) Fryns syndrome: a review of the phenotype and diagnostic guidelines. Am J Med Genet A 124:427–433

    Article  Google Scholar 

  3. Enns GM, Cox VA, Goldstein RB, et al (1998) Congenital diaphragmatic defects and associated syndromes, malformations, and chromosome anomalies: a retrospective study of 60 patients and literature review. Am J Med Genet 79:215–225

    Article  PubMed  CAS  Google Scholar 

  4. Stege G, Fenton A, Jaffray B (2003) Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia. Pediatrics 112:532–535

    Article  PubMed  Google Scholar 

  5. Jaillard S, Pierrat V, Dubois A, et al (2003) Outcome at 2 years of infants with congenital diaphragmatic hernia: a populationbased study. Ann Thorac Surg 75:250–256

    Article  PubMed  Google Scholar 

  6. Rudolph A, Heymann MA (1970) Circulation changes during growth in the fetal lamb. Circ Res 26:289–299

    PubMed  CAS  Google Scholar 

  7. Abman SH, Chatfield BA, Hall SL, McMurtry IF (1990) Role of endothelium-derived relaxing factor during transition of pulmonary circulation at birth. Am J Physiol 259:H1921–H1927

    PubMed  CAS  Google Scholar 

  8. Storme L, Rairigh RL, Parker YA, et al (1999) Potassium channel blockade inhibits shear stress-induced pulmonary vasodilation in the ovine fetus. Am J Physiol 276:L220–L228

    PubMed  CAS  Google Scholar 

  9. Magnenant E, Jaillard S, Deruelle P, et al (2003) Role of the alpha2-adrenoceptors on the pulmonary circulation in the ovine fetus. Pediatr Res 54:44–51

    Article  PubMed  CAS  Google Scholar 

  10. Houfflin-Debarge V, Deruelle P, Jaillard S, et al (2005) Effects of antenatal glucocorticoids on circulatory adaptation at birth in the ovine fetus. Biol Neonate 88:73–78

    Article  PubMed  CAS  Google Scholar 

  11. Bargy F, Beaudoin S, Barbet P (2006) Fetal lung growth in congenital diaphragmatic hernia. Fetal Diagn Ther 21:39–44

    Article  PubMed  CAS  Google Scholar 

  12. Boucherat O, Benachi A, Barlier-Mur AM, et al (2007) Decreased lung fibroblast growth factor 18 and elastin in human congenital diaphragmatic hernia and animal models. Am J Respir Crit Care Med 175:1066–1077

    Article  PubMed  CAS  Google Scholar 

  13. Tenbrinck R, Gaillard JL, Tibboel D, et al (1992) Pulmonary vascular abnormalities in experimentally induced congenital diaphragmatic hernia in rats. J Pediatr Surg 27:862–865

    Article  PubMed  CAS  Google Scholar 

  14. Shehata SM, Tibboel D, Sharma HS, Mooi WJ (1999) Impaired structural remodelling of pulmonary arteries in newborns with congenital diaphragmatic hernia: a histological study of 29 cases. J Pathol 189:112–118

    Article  PubMed  CAS  Google Scholar 

  15. Walsh M, Stork E (2001) Persistent pulmonary hypertension of the newborn. Rational therapy bases on pathophysiology. Clin Perinatol 28:609–627

    Article  PubMed  CAS  Google Scholar 

  16. Vukcevic Z, Coppola CP, Hults C, Gosche JR (2005) Nitrovasodilator responses in pulmonary arterioles from rats with nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg 40:1706–1711

    Article  PubMed  Google Scholar 

  17. Newell MA, Au-Fliegner M, Coppola CP, Gosche JR (1998) Hypoxic pulmonary vasoconstriction is impaired in rats with nitrofen-induced congenital diaphragmatic hernia. J Pediatr Surg 33:1358–1362

    Article  PubMed  CAS  Google Scholar 

  18. Solari V, Piotrowska AP, Puri P (2003) Expression of heme oxygenase-1 and endothelial nitric oxide synthase in the lung of newborns with congenital diaphragmatic hernia and persistent pulmonary hypertension. J Pediatr Surg 38:808–813

    Article  PubMed  Google Scholar 

  19. Shehata SM, Sharma HS, Mooi WJ, Tibboel D (2006) Pulmonary hypertension in human newborns with congenital diaphragmatic hernia is associated with decreased vascular expression of nitric-oxide synthase. Cell Biochem Biophys 44:147–155

    Article  PubMed  CAS  Google Scholar 

  20. de Lagausie P, de Buys-Roessingh A, Ferkdadji L, et al (2005) Endothelin receptor expression in human lungs of newborns with congenital diaphragmatic hernia. J Pathol 205:112–118

    Article  PubMed  Google Scholar 

  21. Tourneux P, Rakza T, Bouissou A, et al (2008) Pulmonary circulatory effects of norepinephrine in newborn infants with persistent pulmonary hypertension (PPHN). J Pediatr, in press

  22. Folkesson HG, Chapin CJ, Beard LL, et al (2006) Congenital diaphragmatic hernia prevents absorption of distal air space fluid in late-gestation rat fetuses. Am J Physiol Lung Cell Mol Physiol 290: L478–L484

    Article  PubMed  CAS  Google Scholar 

  23. Schwartz SM, Vermilion RP, Hirschl RB (1994) Evaluation of left ventricular mass in children with left-sided congenital diaphragmatic hernia. J Pediatr 125:447–451

    Article  PubMed  CAS  Google Scholar 

  24. Karamanoukian HL, O’Toole SJ, Rossman JR, et al (1996) Can cardiac weight predict lung weight in patients with congenital diaphragmatic hernia? J Pediatr Surg 31:823–825

    Article  PubMed  CAS  Google Scholar 

  25. Karamanoukian HL, Glick PL, Wilcox DT, et al (1995) Pathophysiology of congenital diaphragmatic hernia. XI: anatomic and biochemical characterization of the heart in the fetal lamb CDH model, J Pediatr Surg 30:925–929

    Article  PubMed  CAS  Google Scholar 

  26. Takayasu H, Sato H, Sugimoto K, Puri P (2008) Downregulation of GATA4 and GATA6 in the heart of rats with nitrofen-induced diaphragmatic hernia. J Pediatr Surg 43:362–366

    Article  PubMed  Google Scholar 

  27. Guarino N, Puri P (2002) Antenatal dexamethasone enhances endothelin-1 synthesis and gene expression in the heart in congenital diaphragmatic hernia in rats. J Pediatr Surg 37:1563–1567

    Article  PubMed  Google Scholar 

  28. Azarow K, Messineo A, Pearl R, et al (1997) Congenital diaphragmatic hernia: a tale of two cities: the Toronto experience. J Pediatr Surg 32:395–400

    Article  PubMed  CAS  Google Scholar 

  29. Bohn D (2002) Congenital diaphragmatic hernia. Am J Respir Crit Care Med 166:911–915

    Article  PubMed  Google Scholar 

  30. Downard C, Jaksic T, Garza J, et al (2003) Analysis of an improved survival rate for congenital diaphragmatic hernia. J Pediatr Surg 38:729–732

    Article  PubMed  Google Scholar 

  31. Boloker J, Bateman D, Wung JT, Stolar C (2002) Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnia/spontaneous respiration/elective repair. J Pediatr Surg 37:357–366

    Article  PubMed  Google Scholar 

  32. Finer NN, Tierney A, Etches PC, et al (1998) Congenital diaphragmatic hernia: developing a protocolized approach. J Pediatr Surg 33:1331–1337

    Article  PubMed  CAS  Google Scholar 

  33. Sakurai Y, Azarow K, Cutz E, et al (1999) Pulmonary barotrauma in congenital diaphragmatic hernia: a clinicopathological correlation. J Pediatr Surg. 34(12):1813–1817

    Article  PubMed  CAS  Google Scholar 

  34. Desfrere L, Jarreau PH, Dommergues M, et al (2000) Impact of delayed repair and elective high-frequency oscillatory ventilation on survival of antenatally diagnosed congenital diaphragmatic hernia: first application of these strategies in the more ’severe’ subgroup of antenatally diagnosed newborns. Intensive Care Med 26:934–934

    Article  PubMed  CAS  Google Scholar 

  35. Reyes C, Chang LK, Waffarn F, et al (1998) Delayed repair of congenital diaphragmatic hernia with early high-frequency oscillatory ventilation during preoperative stabilization. J Pediatr Surg 33:1010–1014

    Article  PubMed  CAS  Google Scholar 

  36. Boucherat O, Benachi A, Chailley-Heu B, et al (2004) Surfactant maturation is not delayed in human fetuses with diaphragmatic hernia. PLoS Med 4:e237

    Article  Google Scholar 

  37. Lally KP, Lally PA, Langham MR, et al (2004) Surfactant does not improve survival rate in preterm infants with congenital diaphragmatic hernia. J Pediatr Surg 39:829–833

    Article  PubMed  Google Scholar 

  38. Suda K, Bigras JL, Bohn D, et al (2000) Echocardiographic predictors of outcome in newborns with congenital diaphragmatic hernia. Pediatrics 105:1106–1109

    Article  PubMed  CAS  Google Scholar 

  39. Bohn D (2002) Congenital diaphragmatic hernia. Am J Respir Crit Care Med 166:911–915

    Article  PubMed  Google Scholar 

  40. Berman RE, Barst RJ (2006) Pulmonary arterial hypertension: a comprehensive review of pharmacological treatment. Treat Respir Med 5:117–127

    Article  Google Scholar 

  41. Chiu P, HL Hedrick (2008) Postnatal management and long-term outcome for survivors with congenital diaphragmatic hernia. Prenat Diagn 28:592–603

    Article  PubMed  Google Scholar 

  42. Inamura N, A Kubota, T Nakajima, et al (2005) A proposal of new therapeutic strategy for antenatally diagnosed congenital diaphragmatic hernia. J Ped Surg 40:1315–1319

    Article  Google Scholar 

  43. Houfflin Debarge V, Sicot B, Jaillard S, et al (2007) Mechanisms of pain-induced pulmonary vasoconstriction: experimental study in fetal lambs. Anesth Analg 104:799–806

    Article  PubMed  Google Scholar 

  44. Jaillard S, Houfflin-Debarge V, Riou Y, et al (2001) Effects of catecholamines on the pulmonary circulation in the ovine fetus. Am J Physiol 281:R607–R614

    CAS  Google Scholar 

  45. Liet JM, Boscher C, Gras-Leguen C, et al (2002) Dopamine effects on pulmonary artery pressure in hypotensive preterm infants with patent ductus arteriosus J Pediatr 140:373–375

    Article  PubMed  CAS  Google Scholar 

  46. Bouissou A, Rakza T, Klosowski S, et al (2008) Hypotension in preterm infants with significant patent ductus arteriosus: effects of dopamine. J Pediatr, in press

  47. Houfflin-Debarge V, Delelis A, Jaillard S, et al (2005) Effects of nociceptive stimuli on the pulmonary circulation in the ovine fetus. Am J Physiol Regul Integr Comp Physiol 288:R547–R553

    PubMed  CAS  Google Scholar 

  48. Nishina H, Ozaki T, Hanson MA, Poston L (1999) Mechanisms of noradrenaline-induced vasorelaxation in isolated femoral arteries of the neonatal rat. Br J Pharmacol 127:809–812

    Article  PubMed  CAS  Google Scholar 

  49. Tourneux P, Rakza T, Abazine A, et al (2008) Noradrenaline for management of septic shock refractory to fluid loading and dopamine or dobutamine in full-term newborn infants. Acta Paediatr 97:177–180

    Article  PubMed  CAS  Google Scholar 

  50. Dellinger RP, Carlet JM, Masur H, et al (2004) Surviving sepsis campaign guidelines for management of severe sepsis and septic shock. Crit Care Med 32:858–873

    Article  PubMed  Google Scholar 

  51. Jaillard S, Houfflin-Debarge V, Riou Y, et al (2001) Effects of catecholamines on the pulmonary circulation in the ovine fetus. Am J Physiol 281: R607–614

    CAS  Google Scholar 

  52. Jaillard S, Elbaz F, Bresson-Just S, et al (2004) Pulmonary vasodilator effects of norepinephrine during the development of chronic pulmonary hypertension in neonatal lambs. Br J Anaesth 93:818–824

    Article  PubMed  CAS  Google Scholar 

  53. Deruelle P, Houfflin-Debarge V, Magnenant E, et al (2003) Effects of antenatal glucocorticoids on pulmonary vascular reactivity in the ovine fetus. Am J Obstet Gynecol 189:208–215

    Article  PubMed  CAS  Google Scholar 

  54. Jaillard S, Houfflin-Debarge V, Storme L (2003) Higher risk of persistent pulmonary hypertension of the newborn after caesarean section. J Perinat Med 31:538–539

    Article  PubMed  Google Scholar 

  55. Finer NN, Barrington KJ (2006) Nitric oxide for respiratory failure in infants born at or near term. Cochrane Database System Rev (4):CD000399

    Google Scholar 

  56. Archer SL, Huang JM, Hampl V, et al (1994) Nitric oxide and cGMP cause vasorelaxation by activation of a charybdotoxinsensitive K channel by cGMP-dependent protein kinase. Proc Natl Acad Sci USA 91:7583–7587

    Article  PubMed  CAS  Google Scholar 

  57. Larrue B, Jaillard S, Lorthioir M, et al (2005) Pulmonary vascular effects of sildenafil on the development of chronic pulmonary hypertension in the ovine fetus. Am J Physiol Lung Cell Mol Physiol 288:L1193–L2000

    Article  PubMed  CAS  Google Scholar 

  58. Kinsella JP, Ivy DD, Abman SH (2005) Pulmonary vasodilator therapy in congenital diaphragmatic hernia: acute, late, and chronic pulmonary hypertension. Semin Perinatol 29:123–128

    Article  PubMed  Google Scholar 

  59. The Neonatal Inhaled Nitric Oxide Study Group (NINOS) (1997) Inhaled nitric oxide and hypoxic respiratory failure in infants with congenital diaphragmatic hernia. Pediatrics 99:838–845

    Article  Google Scholar 

  60. Kinsella JP (2008) Inhaled NO in term newborns. Early Human Dev 84:709–716

    Article  CAS  Google Scholar 

  61. Larrue B, Jaillard S, Lorthioir M, et al (2005) Pulmonary vascular effects of sildenafil on the development of chronic pulmonary hypertension in the ovine fetus. Am J Physiol Lung Cell Mol Physiol 288: L1193–L2000

    Article  PubMed  CAS  Google Scholar 

  62. Dillon PW, Cilley RE, Hudome SM, et al (1995) Nitric oxide reversal of recurrent pulmonary hypertension and respiratory failure in an infant with CDH after successful ECMO therapy. J Pediatr Surg 30:743–744

    Article  PubMed  CAS  Google Scholar 

  63. Kinsella JP, Parker TA, Ivy DD, Abman SH (2003) Noninvasive delivery of inhaled nitric oxide therapy for late pulmonary hypertension in newborn infants with congenital diaphragmatic hernia. J Pediatr 142:397–401

    Article  PubMed  CAS  Google Scholar 

  64. Sebkhi A, Strange JW, Phillips SC, et al (2003) Phosphodiesterase type 5 as a target for the treatment of hypoxia-induced pulmonary hypertension. Circulation 107:3230–3235

    Article  PubMed  CAS  Google Scholar 

  65. Noori S, Friedlich P, Wong P, et al (2007) Cardiovascular effects of sildenafil in neonates and infants with congenital diaphragmatic hernia and pulmonary hypertension. Neonatology 91:92–100

    Article  PubMed  CAS  Google Scholar 

  66. Keller RL, Moore P, Teitel D, et al (2006) Abnormal vascular tone in infants and children with lung hypoplasia: findings from cardiac catheterization and the response to chronic therapy. Pediatr Crit Care Med 7:589–594

    Article  PubMed  Google Scholar 

  67. Bétrémieux P, Gaillot T, de la Pintière A, et al (2004) Congenital diaphragmatic hernia: prenatal diagnosis permits immediate intensive care with high survival rate in isolated cases. A population-based study. Prenat Diagn 24:487–493

    Article  PubMed  Google Scholar 

  68. Ivy D, Le Cras T, Horan M, Abman S (1998) Increased lung preproET-1 and decreased ETB-receptor gene expression in fetal pulmonary hypertension. Am J Physiol 274:L535–L541

    PubMed  CAS  Google Scholar 

  69. Kavanagh M, Battistini B, Jean S, et al (2001) Effect of ABT-627 (A-147627), a potent selective ET(A) receptor antagonist, on the cardiopulmonary profile of newborn lambs with surgically-induced diaphragmatic hernia. Br J Pharmacol 134:1679–1688

    Article  PubMed  CAS  Google Scholar 

  70. Thébaud B, de Lagausie P, Forgues D, et al (2000) ET(A)-receptor blockade and ET(B)-receptor stimulation in experimental congenital diaphragmatic hernia. Am J Physiol Lung Cell Mol Physiol 278:L923–L933

    PubMed  Google Scholar 

  71. Chiu P, Hedrick HL (2008) Postnatal management and long-term outcome for survivors with congenital diaphragmatic hernia. Prenat Diagn 28:592–603

    Article  PubMed  Google Scholar 

  72. UK Collaborative ECMO Group (1998) The collaborative UK ECMO trial: follow-up to 1 year of age. 101:E1

    Google Scholar 

  73. El Hassani A, Michaud L, Chartier A, et al (2005) Cow’s milk protein allergy after neonatal intestinal surgery. Arch Pediatr 12(2):134–139

    Article  PubMed  Google Scholar 

  74. Bohn D (2002) Congenital diaphragmatic hernia. Am J Respir Crit Care Med 166:911–915

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to L. Storme.

Additional information

Pour le centre de référence « hemie diaphragmatique congénitale »

About this article

Cite this article

Storme, L., Rakza, T., Sfeir, R. et al. Prise en charge médicale per- et postnatale de la hernie congénitale diaphragmatique. Rev. med. perinat. 1, 26–36 (2009). https://doi.org/10.1007/s12611-009-0008-5

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12611-009-0008-5

Mots clés

Keywords

Navigation